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Desmin Polyclonal Antibody, APC Conjugated

Applications

  • IF(ICC)

Reactivity

  • Human
  • Mouse
  • Rat
Overview
Catalog # bs-20702R-APC
Product Name Desmin Polyclonal Antibody, APC Conjugated
Applications IF(ICC)
Reactivity Human, Mouse, Rat
Specifications
Conjugation APC
Host Rabbit
Source KLH conjugated synthetic peptide derived from human Desmin
Immunogen Range 311-400/470
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20¡C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Target
Gene ID 1674
Swiss Prot P17661
Synonyms CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.
Background filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z line structures. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.
Application Dilution
IF(ICC) 1:50-200